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http://purl.uniprot.org/citations/35587511http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/35587511http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/35587511http://www.w3.org/2000/01/rdf-schema#comment"The molecular basis of interindividual clinical variability upon infection with Staphylococcus aureus is unclear. We describe patients with haploinsufficiency for the linear deubiquitinase OTULIN, encoded by a gene on chromosome 5p. Patients suffer from episodes of life-threatening necrosis, typically triggered by S. aureus infection. The disorder is phenocopied in patients with the 5p-(Cri-du-Chat) chromosomal deletion syndrome. OTULIN haploinsufficiency causes an accumulation of linear ubiquitin in dermal fibroblasts, but tumor necrosis factor receptor-mediated nuclear factor κB signaling remains intact. Blood leukocyte subsets are unaffected. The OTULIN-dependent accumulation of caveolin-1 in dermal fibroblasts, but not leukocytes, facilitates the cytotoxic damage inflicted by the staphylococcal virulence factor α-toxin. Naturally elicited antibodies against α-toxin contribute to incomplete clinical penetrance. Human OTULIN haploinsufficiency underlies life-threatening staphylococcal disease by disrupting cell-intrinsic immunity to α-toxin in nonleukocytic cells."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.org/dc/terms/identifier"doi:10.1126/science.abm6380"xsd:string
http://purl.uniprot.org/citations/35587511http://purl.org/dc/terms/identifier"doi:10.1126/science.abm6380"xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Liston A."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Liston A."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Yang R."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Yang R."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Zhang P."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Zhang P."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Wouters C."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Wouters C."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Boisson B."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Boisson B."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Molina H."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Molina H."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Lyonnet S."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Lyonnet S."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Casanova J.L."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Casanova J.L."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Aksentijevich I."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Aksentijevich I."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Kastner D.L."xsd:string
http://purl.uniprot.org/citations/35587511http://purl.uniprot.org/core/author"Kastner D.L."xsd:string